Abstracts tagged "interferon"

Abstract Number: 1658 • ACR Convergence 2023
Title: Inflammatory Arthritis Across the Age-Spectrum: Single-Cell Profiling of the Inflamed Synovium in Children with Juvenile Idiopathic Arthritis
Chrissy Bolton¹, Christopher Mahony², Charlotte Smith², Vicky Alexiou³, Huong Nguyen³, Patricia Reis-Nisa², Søren Lomholt⁴, Annie Hackland², Sugrah Sultan⁵, Klaudia Kupiec³, Sunit Davda⁶, Charlene Foley⁶, Calliope Dendrou¹, Elizabeth C Rosser⁷, Accelerating Medicines Partnership Program RA SLE Network⁸, Anna Helena Jonsson⁹, Fan Zhang¹⁰, Michael Brenner¹¹, Soumya Raychaudhuri⁹, Christopher Buckley¹, Manigandan Thyagarajan⁵, Zishan Shiekh⁵, Sandrine Compeyrot-Lacassagne⁶, Samantha Chippington⁶, Mark Coles¹, Eslam Al-Abadi⁵, Andrew Filer², Tissue Research in Childhood Onset Inflammatory Arthritis (TRICIA) Consortium¹², Lucy R Wedderburn³ and Adam Croft², ¹University of Oxford, Oxford, United Kingdom, ²University of Birmingham, Birmingham, United Kingdom, ³UCL Great Ormond Street Institute of Child Health, London, United Kingdom, ⁴Aarhus University, Aarhus, Denmark, ⁵Birmingham Women’s and Children’s NHS Foundation Trust, Birmingham, United Kingdom, ⁶Great Ormond Street Hospital, London, United Kingdom, ⁷University College London, London, United Kingdom, ⁸Cedars-Sinai Medical Center, Los Angeles, CA, ⁹Brigham and Women's Hospital, Boston, MA, ¹⁰University of Colorado, Aurora, CO, ¹¹Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ¹²MRC, Birmingham, United Kingdom
Background/Purpose: Understanding the unique and shared pathogenic processes between childhood-onset and adult-onset inflammatory arthritides is needed to guide more effective drug development and their application…
Abstract Number: 0010 • ACR Convergence 2023
A Potential Mechanism for Major Adverse Cardiac Events Associated with JAK Inhibitors: JAK Inhibitor Withdrawal Causes Urokinase Release by Primed STAT Signaling
Sara McCoy¹, Ilya Gurevic² and jacques Galipeau², ¹University of Wisconsin School of Medicine and Public Health, Middleton, WI, ²University of Wisconsin, Madison, WI
Background/Purpose: Sjögren’s Disease (SjD) has high glandular IFNg levels, associated with disease activity and lymphoma risk. We showed that IFNg-stimulated minor salivary gland (SG)-mesenchymal stromal…
Abstract Number: 0886 • ACR Convergence 2023
Neonatal Roseolovirus Infection Predisposes to Development of Lupus-like Disease After TLR7 Stimulation
Tarin Bigley¹, Eden Xue¹, Lifei Zhu¹, Li-ping Yang¹ and wayne Yokoyama², ¹Washington University in St. Louis, St. Louis, MO, ²Washington University School of Medicine, St. Louis, MO
Background/Purpose: Viral infections have been implicated as major factors in autoimmune disease but demonstrating causality is often challenging. We found that neonatal infection with a…
Abstract Number: 1660 • ACR Convergence 2023
Still’s Disease Patients with High Interferon-stimulated Gene Expression Have Enrichment of Rare, de Novo and Recessive Protein Altering Variants in Innate Immune Pathways
Mariana Correia Marques¹, Zuoming Deng², Navid Chowdhury², Elizabeth Schmitz³, Alana Platukus⁴, Stephen Brooks⁵, Carol Lake², Ly-Lan Bergeron², Michelle Millwood² and Michael Ombrello⁶, ¹National Institute of Arthritis & Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ²National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ³National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), St. Louis, MO, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Philadelphia, PA, ⁵Biodata Mining and Discovery Section, National Institute of Arthritis and Musculoskeletal and Skins Diseases, National Institutes of Health, Bethesda, MD, ⁶National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), North Bethesda, MD
Background/Purpose: Still’s disease (systemic juvenile idiopathic arthritis in children, adult-onset Still’s disease in adults) is an enigmatic inflammatory condition that affects people of all ages.…
Abstract Number: 0055 • ACR Convergence 2023
Elevated LINE-1 Expression in SLE Keratinocytes Leads to LINE-1 Reverse Transcriptase-dependent Type I Interferon Responses
Wenyan Miao¹, Daniel Rios¹, Mehrnaz Gharaee-Kermani², Craig Dobry², Artur Jaroszewicz¹, Cedric Arisdakessian¹, Enrique Garcia-Rivera¹, Nafeeza Hafeez¹, Brian Desrosiers¹, Jess Floro¹, Jared Steranka¹, Menachem Fromer¹, Dennis Zaller¹ and J. Michelle Kahlenberg², ¹Rome Therapeutics, Boston, MA, ²University of Michigan, Ann Arbor, MI
Background/Purpose: Long Interspersed Element-1 (LINE-1) are retrotransposable DNA elements that make up ~17% of the human genome, and their role in health and disease is…
Abstract Number: 0891 • ACR Convergence 2023
Selective and Potent Inhibition of Cyclic GMP-AMP Synthase (cGAS) Fully Normalizes Autoinflammation Across Tissues in a Trex1-/- Mouse Model for Type I Interferonopathies
Arnaud Bourin¹, Zuzana Hořejší², Isabelle Cambré¹, Juraj Dobiaš², Mikhail Klychnikov², Radek Liboska², Maroš Smolíček², Zdeněk Vavřina², Wanda Haeck¹, Hugo Klaassen¹, Marnik Nijs¹, Kristine Metzger¹, Sandro Boland¹, Dries De Clercq¹, Sara Allasia¹, Gunter Carlens¹, Patrick Chaltin³, Arnaud Marchand¹, Ondřej Páv², Matthias Versele¹ and Gabriel Birkuš², ¹Centre for Drug Design and Discovery (CD3) - Cistim, Leuven, Belgium, ²Institute of Organic Chemistry and Biochemistry, Prague, Czech Republic, ³Centre for Drug Design and Discovery (CD3) - KU Leuven, Leuven, Belgium
Background/Purpose: The cGAS enzyme is a sensor of cytosolic double-strand (ds) DNA. It serves to detect viruses and elicits an acute, transient cGAMP-STING mediated type…
Abstract Number: 1664 • ACR Convergence 2023
Fibroblast Specific Interferon Regulatory Factor 7 (IRF7) Expression Is a Key Link Between Type I Interferon Activation and the Exaggerated Dermal and Pulmonary Fibrosis in Systemic Sclerosis
Minghua Wu¹, Jerry Alonso², Julio charles², Brian Skaug², Tingting Mills³, Maureen Mayes² and Shervin Assassi⁴, ¹Department of Internal Medicine, Division of Rheumatology, The University of Texas Health Science Center at Houston, Houston, TX, ²Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, ³Biochemistry Department, University of Texas McGovern Medical School, Houston, TX, ⁴University of Texas McGovern Medical School at Houston, Houston, TX
Background/Purpose: A prominent interferon (IFN) activation signature has been observed at the peripheral blood and end-organ levels in systemic sclerosis (SSc). However, the mechanisms by…
Abstract Number: 0056 • ACR Convergence 2023
The Activation of cGAS-STING Pathway in Systemic Lupus Erythematosus
Jie An¹, Stephen Wilson², Jill Henault³ and Keith Elkon¹, ¹University of Washington, Seattle, WA, ²Bristol Myers Squibb, Cambridge, MA, ³Bristol Myers Squibb, Acton, MA
Background/Purpose: The majority of patients with SLE show a striking Type I Interferon (IFN-I) signature in their peripheral blood. Although this signature can be generated…
Abstract Number: 0893 • ACR Convergence 2023
Involvement of Type I Interferon-responsive Myeloid Cells in Renal Inflammation in a Lupus Mouse Model
Trine Jorgensen and Lindsey Han, Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, OH
Background/Purpose: Systemic Lupus Erythematosus (SLE) is an autoimmune disease that can cause damage to multiple organs, including the kidneys in Lupus Nephritis (LN). Current treatments…
Abstract Number: 1665 • ACR Convergence 2023
Endothelial Response to Type I Interferon Contributes to Vasculopathy and Fibrosis and Predicts Disease Progression of Systemic Sclerosis
Hanlin Yin¹, Oliver Distler², Bin Li³, Qingran Yan⁴ and Liangjing Lu¹, ¹Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China, ²Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ³Shanghai institute of immunology, Shanghai, China, ⁴Department of Rheumatology, Renji Hospital, Shanghai Jiaotong Univeisty School of medcine, Shanghai, China
Background/Purpose: Type I interferon (IFN-1) signature is a hallmark of patients with systemic sclerosis (SSc). However, its significance in clinical stratification and contribution to deterioration…
Abstract Number: 0091 • ACR Convergence 2023
Impaired X-Chromosome Inactivation Maintenance in T Cells Is Associated with Features of Reduced Disease Severity in a Toll-Like Receptor 7-Driven Model of Systemic Autoimmunity
Nikhil Jiwrajka¹, Zowie Searcy², Claudia Lovell², Natalie Toothacre², Katherine Forsyth² and Montserrat Anguera², ¹Divison of Rheumatology, Department of Medicine, Hospital of the University of Pennsylvania, Phildelphia, PA, ²Department of Biomedical Sciences, University of Pennsylvania School of Veterinary Medicine, Philadelphia, PA
Background/Purpose: Many systemic autoimmune rheumatic diseases, including systemic lupus erythematosus (SLE), Sjögren's syndrome, and systemic sclerosis are highly female-biased. Although these diseases are more prevalent…
Abstract Number: 0894 • ACR Convergence 2023
The Cellular and Spatial Type I Interferon Response Following Skin Exposure to Ultraviolet Light
Jie An¹, Xizhang Sun¹, Rayan Najjar¹, Connie Zhao¹, Paul Kong², Stephanie Weaver², Amanda Koehne², Matt Fitzgibbon² and Keith Elkon¹, ¹University of Washington, Seattle, WA, ²Fred Hutchinson Cancer Center, Seattle, WA
Background/Purpose: SLE patients characteristically have a prominent type I interferon (IFN-I) signature in lesional and non-lesional skin. We recently demonstrated that, following a single exposure…
Abstract Number: 1708 • ACR Convergence 2023
In Cis SOCS1 Variants Illustrate the Precise Regulation of Interferon Signaling Needed to Prevent Autoimmunity
Yan Du¹, Evan Hsu², Kailey Brodeur³, Meng Liu⁴, Mindy Lo⁵, Craig Platt³ and Pui Lee⁵, ¹Harvard medical school, Boston, MA, ²Wesleyan University, Newton, MA, ³Boston Children's Hospital, Boston, MA, ⁴Southern Medical University, Boston, MA, ⁵Division of Immunology, Boston Children's Hospital, Boston, MA
Background/Purpose: Systemic autoimmunity can be driven by monogenic or polygenic risk variants. We aimed to characterize the genetic basis of disease in a family with…
Abstract Number: 0093 • ACR Convergence 2023
Single Cell RNA-seq and Mass Cytometry Reveal a Cytotoxic CD8 Effector T Cell Population Associated with Interstitial Lung Disease in Systemic Sclerosis Patients
Ye Cao¹, Takanori Sasaki², Richard Ainsworth³, Kim Taylor⁴, Nunzio Bottini⁵, Mehreen Elahee⁶, Edy Kim⁷, Francesco Boin³ and Deepak Rao⁷, ¹Brigham and Women's Hospital, Boston, MA, ²Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ³Cedars-Sinai Medical Center, Los Angeles, CA, ⁴University of California San Francisco, San Francisco, CA, ⁵University of California, San Francisco, CA, ⁶University of Pittsburgh Medical Center, Pittsburgh, PA, ⁷Brigham and Women's Hospital, Boston, MA
Background/Purpose: Interstitial lung disease (ILD) is a major cause of morbidity and mortality in systemic sclerosis (SSc). We aimed to identify features of circulating immune…
Abstract Number: 0910 • ACR Convergence 2023
17β-estradiol and B-cell Intrinsic Type I Interferon Amplify Toll-like Receptor 7 Signaling Loop in B Cells of Female Lupus Prone BXD2 Mice
Kathryn Sullivan, Yong Wang, Winn Chatham, John Mountz and Hui-chen Hsu, University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: While systemic lupus erythematosus (SLE) disproportionally affects women versus men, elevation in estradiol (E2) alone is not sufficient to promote the development of autoantibody…

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